Mitochondrial Functions and Oxidative Stress in ALS Patients

In Amyotrophic Lateral Sclerosis (ALS), malnutrition is frequent (16 to 50 % of the patients) and is an independent prognostic factor. One of the implicated factors is the increase of resting energy expenditure (REE) which can be found in about 50 % of ALS patients. The origin of this hypermetabolism is currently unknown but could be located in the mitochondria. In fact, some studies have found mitochondrial abnormalities and the existence of an oxidative stress. Thus, the aim of this study is to characterize the mitochondrial abnormalities and the oxidant/antioxidant status of ALS patients and to determine their relationship with the metabolic status, hypermetabolism or normometabolism. Three groups of patients will be studied : 20 hypermetabolic ALS patients, 20 normometabolic ALS patients and 20 healthy volunteers paired for age and sex.